Case 1 Case 2 Initial BP (mmHg) 200/120 208/131 Presenting symptoms Erythromelalgia, occipital headaches, Vertigo, one episode of dyspnoea + eye floaters + syncope (1-2 minutes) Acrocyanosis, Vasculitic skin rashes, nonhealing toe ulceration Past medical history Spondylolisthesis Migraines with aura Family history Father: Vasovagal syncope, myocardial infarction Unremarkable Social history Ex-smoker 20 units of alcohol per week Investigations and results Fundus examination Papilloedema with nasal margin blurring Arteriovenous nipping with nasal margin blurring Urine dipstick Unremarkable Unremarkable FBC WBC— PLT -1096 x109 /L Liver function tests Not indicated Total bilirubin—38 μ mol/L; ALT—42 U/L; Gamma GT—94 U/L Electrocardiogram Sinus rhythm 70 bpm, left axis deviation, biphasic T waves in leads V5 and V6 Unremarkable Echocardiogram Moderate global left ventricular hypertrophy with 1.4 cm wall thickness Mild aortic dilatation Creatinine 146 μ M 88 μ M Renin 107 mU/L 80 mU/L Autoimmune screen Negative Negative Vasculitic skin rash biopsy N/A Unremarkable Contrast CT abdomen N/A Enlarged left adrenal gland, lower abdominal lymphadenopathy Inert gas rebreathing studies (clinical research facility) N/A Peripheral vascular resistance5 Interventions Renal artery angioplasty, aspirin 75 mg, intermittent venesection, interferon-alpha 2A Aspirin 75 mg, long-acting nifedipine Outcomes Normalisation of BP and renal parameters, minor side effects from interferon therapy (decreased libido and reported thinning of hair), now managed with single dose of long-acting nifedipine Normalisation of BP, no adverse outcomes reported, toe ulceration now healed
